title: Therapeutic cytodifferentiation in alveolar rhabdomyosarcoma  without genetic change of the PAX3-FKHR chimeric fusion gene:  a case study.
abstract: Alveolar rhabdomyosarcoma (ARMS) is a subtype of rhabdomyosarcoma and usually occurs in childhood and adolescence. ARMS is characterized by its aggressive behavior and poor prognosis. To improve the unfavorable prognosis, new therapeutic developments and the establishment of methods for precise prognostic prediction are required. We describe a case of ARMS, solid variant, which occurred in a 10-year-old boy. After chemotherapy and radiotherapy, the tumor morphologically and immunohistochemically showed marked cytodifferentiation, whereas the exact same PAX3-FKHR chimeric fusion gene transcript was detected in samples before and after treatment. The result of this study seems to indicate that therapeutic cytodifferentiation does not always correlate with genetic change and favorable prognosis in ARMS.
authors.forename: Michiyuki
authors.initials: M
authors.lastname: Hakozaki
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hojo
authors.forename: Takahiro
authors.initials: T
authors.lastname: Tajino
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Yamada
authors.forename: Shinichi
authors.initials: S
authors.lastname: Kikuchi
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kikuta
authors.forename: Shinichi
authors.initials: S
authors.lastname: Konno
authors.forename: Masafumi
authors.initials: M
authors.lastname: Abe
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002081:Buttocks; D002471:Cell Transformation, Neoplastic; D059248:Chemoradiotherapy; D002648:Child; D003131:Combined Modality Therapy; D017809:Fatal Outcome; D000071161:Forkhead Box Protein O1; D051858:Forkhead Transcription Factors / Q000235:genetics; D050939:Gene Fusion / Q000235:genetics; D006801:Humans; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D000072041:PAX3 Transcription Factor; D051761:Paired Box Transcription Factors / Q000235:genetics; D036102:Peripheral Blood Stem Cell Transplantation; D012186:Retroperitoneal Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy*; D018232:Rhabdomyosarcoma, Alveolar / Q000235:genetics / Q000473:pathology* / Q000628:therapy*
chemical_list: C085126:FOXO1 protein, human; D000071161:Forkhead Box Protein O1; D051858:Forkhead Transcription Factors; D015514:Oncogene Proteins, Fusion; D000072041:PAX3 Transcription Factor; C489409:PAX3 protein, human; D051761:Paired Box Transcription Factors